Purpose: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. It has a poor prognosis, with published 5-year overall survival between 15 to 36%. There are cranial and extra-cranial forms of MRT and extra-cranial MRT can be subdivided into renal and soft tissue MRT. Due to its\' extreme rarity, most of the available data is based on retrospective case series. To add to the current knowledge of this disease, we reviewed the children treated for extra-cranial MRT in our institute. Materials and methods: A retrospective medical record review was done on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children’s Hospital between January 2003 and July 2013. Results: Ten children (7 boys, 3 girls) were diagnosed with extra-cranial MRT at median age of 8 months old (range, 3 to 159 months). Six patients (60%) were diagnosed during infancy, but a child was diagnosed at 13.2 years of age. Six patients (60%) had renal MRT and 4 (40%) had soft tissue MRT in submental, paraspinal, retrosternal or coccygeal area. Five patients (50%) had metastasis at diagnosis; 3 patients had metastases to sites that included the lungs and 2 patients had metastases but did not have lung metastases. Patients received multimodal therapies. The entire cohort received chemotherapy and 3 patients (30%) received additional high dose chemotherapy with autologous stem cell rescue after intravenous melphalan, etoposide and carboplatin conditioning. Surgical resections were feasible in 7 patients (70%); 3 patients had upfront surgery and 4 had delayed surgery. Therapeutic irradiations were performed in 5 patients (50%). Five patients (50%) had events; 3 patients progressed and 2 patients relapsed. The overall survival of the cohort was 50.0% with median follow-up duration of 25 months (range, 3 to 112 months). Children with lung metastasis had significantly worse survival compared to those with metastasis to sites other than the lungs or those without metastasis; OS 0% versus 50.0% versus 80.0% ( P=0.003). Conclusion: Our study reaffirmed the highly aggressive nature of extra-cranial MRT, but showed a slight improvement in survival compared to previous reports. Children with lung metastasis were shown to have substantially worse outcome. Devising treatment strategies for lung metastasis is thus imperative.